Osteosarcoma does not often affect the bones of the skull, occurring preferentially in the appendicular skeleton. The patient's age at onset seems to be later when the tumor is in the skull than in other sites. CT and MRI are at present the best means of establishing the extent of the tumor. Surgical removal of the lesion combined with polychemotherapy is the basis of treatment. We report a case of osteosarcoma of the skull in a child and review relevant publications.